Department of Pediatrics

Education

• MD, University of Buenos Aires, 1990

Training

• Pediatric Residency, Hospital Nacional de Pediatría “Juan P. Garrahan”, 1991 - 1994
• Pediatric Hematology-Oncology Fellowship, Children’s Hospital of Orange County, 1995 - 1997
• Pediatric Residency 2nd & 3rd years, Children’s Hospital of Orange County, 1997 - 1999
• Hematology-Oncology Fellowship 3rd year, Children’s Hospital of Orange County, 1999 - 2000

Licensure and Board Certification

• CA, California Medical License
• IA, Iowa Medical License
• CO, Colorado Medical License
• 2000 - PresAmerican Board of Pediatric (Hematology & Oncology)
• 2020 - PresMO, Missouri Medical License

Honors and Awards

• Scholar, Children’s Health Research Center (CHRC-K12-NIH) University of Iowa, 2001 - 2003
• Hemophilia and Thrombosis Research Society Investigator in Hemostasis Award, 2003
• James C. Taylor Teaching Award, University of Iowa, Department of Pediatrics, 2003
• The Inaugural Postle Family Chair in Pediatric Cancer and Blood Disorders, Children's Hospital Colorado, 2008 - 2019
• Best Doctors in America, 2009
• Vice-President, President Elect: Hemostasis and Thrombosis Research Society (HTRS), 2010
• Best Doctors in America, 2011
• Best Doctors in America, 2012
• Excellence in Mentoring and Advising Award, Research/Scholarship, University of Colorado School of Medicine, 2012
• Chair, Von Willebrand Factor Subcommittee, International Society of Thrombosis and Hemostasis (ISTH), 2013 - 2016
• Frank Oski Memorial Lectureship Award, American Society of Pediatric Hematology Oncology, 2013
• Chair, State of the Art Symposium, American Society of Hematology (ASH), 2014
• Co-Vice Chair, Gordon Research Conference in Hemostasis, 2014
• Elected Member-At-Large of the Leadership Committee of the Arteriosclerosis Thrombosis and Vascular Biology (ATVB) Council of the American Heart Association (AHA), 2014
• Arthur R. Thompson Inaugural Lectureship, Puget Sound Blood Center, University of Washington, 2015
• Best Doctors in America, 2015
• Co-Chair (with James Morrissey, PhD), Gordon Research Conference in Hemostasis, 2016
• Co-Chair (with Selina Luger, MD), Education Program American Society of Hematology Annual 58th Annual Meeting, 2016
• Scientific Advisory Board, National Rare Diseases Center (CIBERER), Spain, 2016 - Pres
• Visiting Professor, Aflac Cancer and Blood Disorders Center Fall Research Conference, Emory University, 2016
• Best Doctors in America, 2017
• Elected Member, American Association of Physicians (AAP), 2017
• Elected Member International Society of Thrombosis and Haemostasis (ISTH) Council, Class of 2024, 2018
• Elected Vice President (President Elect) American Society of Pediatric Hematology Oncology (ASPHO), 2018
• Ralph Gruppo Inaugural Lectureship, Cincinnati Children's Hospital, 2018
• Elizabeth Finney McDonnell Endowed Chair in Pediatric Hematology Oncology, Washington University in St. Louis, 2020 - Pres
• Elected Member American Pediatric Society, 2020

Recent Publications view all (129)

Publication Co-Authors

Megan A. Cooper, M.D., Ph.D.

Brooke Sadler

David Bark

Julia Therese Warren, M.D., Ph.D.

Washington University has a long tradition of collaboration amongst colleagues.The faculty above have co-authored publications with Jorge Di Paola

1. Christopherson PA, Haberichter SL, Flood VH, Perry CL, Sadler BE, Bellissimo DB, Di Paola J, Montgomery RR, Zimmerman Program Investigators.. Molecular pathogenesis and heterogeneity in type 3 VWD families in U.S. Zimmerman Program. J Thromb Haemost. 2022. PMID:35343054 
2. Warren JT, Di Paola J. Genetics of Inherited thrombocytopenias. Blood. 2022. PMID:35167650 
3. Ng CJ, Liu A, Venkataraman S, Ashworth K, Baker CD, O'Rourke R, Vibhakar R, Jones K, Di Paola J. Single Cell Transcriptional Analysis of Human Endothelial Colony Forming Cells from Patients with Low VWF Levels. Blood. 2022. PMID:35143643 
4. Sadler B, Minard CG, Haller G, Gurnett CA, O'Brien SH, Wheeler A, Jain S, Sharma M, Zia A, Kulkarni R, Mullins E, Ragni MV, Sidonio R, Dietrich JE, Kouides PA, Di Paola J, Srivaths L. Whole-exome analysis of adolescents with low VWF and heavy menstrual bleeding identifies novel genetic associations. Blood Adv. 2022;6(2):420-428. PMCID:PMC8791588  PMID:34807970 
5. Sake CL, Metcalf AJ, Meagher M, Di Paola J, Neeves KB, Boyle NR. Isotopically nonstationary ¹³C metabolic flux analysis in resting and activated human platelets. Metab Eng. 2022;69:313-322. PMID:34954086 
6. Kalot MA, Husainat N, Tayiem S, El Alayli A, Dimassi AB, Diab O, Abughanimeh O, Madoukh B, Qureini A, Ameer B, Di Paola J, Eikenboom JCJ, Jacobs-Pratt V, McLintock C, Montgomery R, O'Donnell JS, Sidonio R, Brignardello-Petersen R, Flood V, Connell NT, James PD, Mustafa RA. Bleeding assessment tools in the diagnosis of VWD in adults and children: a systematic review and meta-analysis of test accuracy. Blood Adv. 2021;5(23):5023-5031. PMID:34597385 
7. Perez Botero J, Di Paola J. Diagnostic approach to the patient with a suspected inherited platelet disorder: Who and how to test. J Thromb Haemost. 2021;19(9):2127-2136. PMID:34347927 
8. Mazer MB, Turnbull IR, Miles S, Blood TM, Sadler B, Hess A, Botney MD, Martin RS, Bosanquet JP, Striker DA, Anand NS, Morre M, Caldwell CC, Brakenridge SC, Moldawer LL, Di Paola JA, Hotchkiss RS, Remy KE. Interleukin-7 Reverses Lymphopenia and Improves T-Cell Function in Coronavirus Disease 2019 Patient With Inborn Error of Toll-Like Receptor 3: A Case Report. Crit Care Explor. 2021;3(7):e0500. PMCID:PMC8322565  PMID:34345826 
9. Griffin MT, Ashworth K, Hill N, von Behren J, Di Paola J, Ku DN. Negatively charged nanoparticles of multiple materials inhibit shear-induced platelet accumulation. Nanomedicine. 2021;35:102405. PMID:33932591 
10. Smith N, Warren BB, Smith J, Jacobson L, Armstrong J, Kim J, Di Paola J, Manco-Johnson M. Antithrombin deficiency: A pediatric disorder. Thromb Res. 2021;202:45-51. PMID:33725558 
11. Sadler B, Christopherson P, Haller G, Montgomery RR, Di Paola J, Investigators TZP. VWF antigen levels are associated with burden of rare nonsynonymous variants in the VWF gene. Blood. 2021. PMID:33556167 
12. Connell NT, James PD, Brignardello-Petersen R, Abdul-Kadir R, Ameer B, Arapshian A, Couper S, Di Paola J, Eikenboom J, Giraud N, Grow JM, Haberichter S, Jacobs-Pratt V, Konkle BA, Kouides P, Laffan M, Lavin M, Leebeek FWG, McLintock C, McRae S, Montgomery R, O'Brien SH, O'Donnell JS, Ozelo MC, Scappe N, Sidonio R, Tosetto A, Weyand AC, Kalot MA, Husainat N, Mustafa RA, Flood VH. von Willebrand disease: proposing definitions for future research. Blood Adv. 2021;5(2):565-569. PMCID:PMC7839375  PMID:33496750 
13. Ross JE, Zhang BM, Lee K, Mohan S, Branchford BR, Bray P, Dugan SN, Freson K, Heller PG, Kahr WHA, Lambert MP, Luchtman-Jones L, Luo M, Perez Botero J, Rondina MT, Ryan G, Westbury S, Bergmeier W, Di Paola J. Specifications of the variant curation guidelines for ITGA2B/ITGB3: ClinGen Platelet Disorder Variant Curation Panel. Blood Adv. 2021;5(2):414-431. PMCID:PMC7839359  PMID:33496739 
14. James PD, Connell NT, Ameer B, Di Paola J, Eikenboom J, Giraud N, Haberichter S, Jacobs-Pratt V, Konkle B, McLintock C, McRae S, R Montgomery R, O'Donnell JS, Scappe N, Sidonio R, Flood VH, Husainat N, Kalot MA, Mustafa RA. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv. 2021;5(1):280-300. PMCID:PMC7805340  PMID:33570651 
15. Doherty D, Lavin M, O'Sullivan JM, Ryan K, O'Connell NM, Dougall A, Byrne M, Rafferty M, Doyle MM, Di Paola J, James PD, O'Donnell JS. Management of elective procedures in low von Willebrand factor patients in the LoVIC study. J Thromb Haemost. 2020. PMID:33346399 
16. Delaney C, Davizon-Castillo P, Allawzi A, Posey J, Gandjeva A, Neeves K, Tuder RM, Di Paola J, Stenmark KR, Nozik ES. Platelet activation contributes to hypoxia-induced inflammation Am J Physiol Lung Cell Mol Physiol. 2020. doi:10.1152/ajplung.00519.2020  PMID:33264579 
17. Porter CC, Di Paola J, Pencheva B, Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, Porter CC, Di Paola J, Pencheva B. ETV6 Thrombocytopenia and Predisposition to Leukemia 2020. PMID:33226740 
18. Fisher MH, Kirkpatrick GD, Stevens B, Jones C, Callaghan M, Rajpurkar M, Fulbright J, Cooper MA, Rowley J, Porter CC, Gutierrez-Hartmann A, Jones K, Jordan C, Pietras EM, Di Paola J. ETV6 germline mutations cause HDAC3/NCOR2 mislocalization and upregulation of interferon response genes. JCI Insight. 2020;5(18). PMID:32841218 
19. Gupta S, Konradt C, Corken A, Ware J, Nieswandt B, Di Paola J, Yu M, Wang D, Nieman MT, Whiteheart SW, Brass LF. Hemostasis vs. homeostasis: Platelets are essential for preserving vascular barrier function in the absence of injury or inflammation. Proc Natl Acad Sci U S A. 2020. PMID:32929010 
20. Bortot M, Sharifi A, Ashworth K, Walker F, Cox A, Ruegg K, Clendenen N, Neeves KB, Bark D Jr, Di Paola J. Pathologic Shear and Elongation Rates Do Not Cause Cleavage of Von Willebrand Factor by ADAMTS13 in a Purified System. Cell Mol Bioeng. 2020;13(4):379-390. PMCID:PMC7479076  PMID:32952737 
21. Kim DA, Ashworth KJ, Di Paola J, Ku DN. Platelet α-granules are required for occlusive high-shear-rate thrombosis. Blood Adv. 2020;4(14):3258-3267. PMCID:PMC7391145  PMID:32697818 
22. Park I, Johnson LK, Cox A, Branchford BR, Di Paola J, Bublil EM, Majtan T. Hypermethioninemia Leads to Fatal Bleeding and Increased Mortality in a Transgenic I278T Mouse Model of Homocystinuria. Biomedicines. 2020;8(8). PMCID:PMC7459533  PMID:32722248 
23. Di Paola J, Fisher MH. ETV6-related thrombocytopenia and platelet dysfunction. Platelets. 2020;1-3. PMID:32406789 
24. Botero JP, Lee K, Branchford BR, Bray PF, Freson K, Lambert MP, Luo M, Mohan S, Ross JE, Bergmeier W, Di Paola J, ClinGen Platelet Disorder Variant Curation Expert Panel.. Glanzmann thrombasthenia: genetic basis and clinical correlates. Haematologica. 2020;105(4):888-894. PMCID:PMC7109743  PMID:32139434 
25. Davizon-Castillo P, Allawzi A, Sorrells M, Fisher S, Baltrunaite K, Neeves K, Nozik-Grayck E, DiPaola J, Delaney C. Platelet activation in experimental murine neonatal pulmonary hypertension Physiol Rep. 2020. doi:10.14814/phy2.14386  PMID:32163236 
26. Kalot MA, Al-Khatib M, Connell NT, Flood V, Brignardello-Petersen R, James P, Mustafa RA, VWD working group.. An international survey to inform priorities for new guidelines on von Willebrand disease. Haemophilia. 2019. PMID:31769905 
27. Majtan T, Park I, Cox A, Branchford BR, di Paola J, Bublil EM, Kraus JP. Behavior, body composition, and vascular phenotype of homocystinuric mice on methionine-restricted diet or enzyme replacement therapy. FASEB J. 2019;33(11):12477-12486. PMCID:PMC6902697  PMID:31450979 
28. Link KG, Stobb MT, Sorrells MG, Bortot M, Ruegg K, Manco-Johnson MJ, Di Paola JA, Sindi SS, Fogelson AL, Leiderman K, Neeves KB. A mathematical model of coagulation under flow identifies factor V as a modifier of thrombin generation in hemophilia A. J Thromb Haemost. 2019. PMID:31562694 
29. Bortot M, Ashworth K, Sharifi A, Walker F, Crawford NC, Neeves KB, Bark D Jr, Di Paola J. Turbulent Flow Promotes Cleavage of VWF (von Willebrand Factor) by ADAMTS13 (A Disintegrin and Metalloproteinase With a Thrombospondin Type-1 Motif, Member 13). Arterioscler Thromb Vasc Biol. 2019;39(9):1831-1842. PMID:31291760 
30. Davizon-Castillo P, McMahon B, Aguila S, Bark D, Ashworth K, Allawzi A, Campbell RA, Montenont E, Nemkov T, D'Alessandro A, Clendenen N, Shih L, Sanders NA, Higa K, Cox A, Padilla-Romo Z, Hernandez G, Wartchow E, Trahan GD, Nozik-Grayck E, Jones K, Pietras EM, DeGregori J, Rondina MT, Di Paola J. TNF-α-driven inflammation and mitochondrial dysfunction define the platelet hyperreactivity of aging. Blood. 2019;134(9):727-740. PMCID:PMC6716075  PMID:31311815 
31. Di Paola J, Porter CC. ETV6-related thrombocytopenia and leukemia predisposition. Blood. 2019;134(8):663-667. PMCID:PMC6706811  PMID:31248877 
32. Ward CM, Andrews RK, ISTH State of the Art Speakers.. Illustrated State-of-the-Art Capsules of the ISTH 2019 Congress in Melbourne, Australia. Res Pract Thromb Haemost. 2019;3(3):431-497. PMCID:PMC6611378  PMID:31294333 
33. Hernandez G, Mills TS, Rabe JL, Chavez JS, Kuldanek S, Kirkpatrick G, Noetzli L, Jubair WK, Zanche M, Meyers JR, Stevens BM, Fleenor CJ, Adane B, Dinarello CA, Ashton J, Jordan CT, Di Paola J, Hagman JR, Holers VM, Kuhn KA, Pietras EM. Pro-inflammatory cytokine blockade attenuates myeloid expansion in a murine model of rheumatoid arthritis. Haematologica. 2019. PMID:31101752 
34. Aguila S, Lavin M, Dalton N, Patmore S, Chion A, Trahan GD, Jones KL, Keenan C, Brophy TM, O'Connell NM, Ryan K, Byrne M, Nolan M, Patel A, Preston RJS, James P, Di Paola J, O'Sullivan JM, O'Donnell JS. Increased galactose expression and enhanced clearance in patients with low von Willebrand factor. Blood. 2019;133(14):1585-1596. PMID:30770394 
35. Hu Z, Lavik KI, Liu Y, Vo AH, Richter CE, Di Paola J, Shavit JA. Loss of fibrinogen in zebrafish results in an asymptomatic embryonic hemostatic defect and synthetic lethality with thrombocytopenia. J Thromb Haemost. 2019;17(4):607-617. PMCID:PMC6443434  PMID:30663848 
36. Riemondy KA, Ransom M, Alderman C, Gillen AE, Fu R, Finlay-Schultz J, Kirkpatrick GD, Di Paola J, Kabos P, Sartorius CA, Hesselberth JR. Recovery and analysis of transcriptome subsets from pooled single-cell RNA-seq libraries. Nucleic Acids Res. 2019;47(4):e20. PMCID:PMC6393243  PMID:30496484 
37. Di Paola J, Bray PF. One over PAR or one under PAR: vive la différence. Blood. 2018;132(19):2007-2008. PMCID:PMC6236467  PMID:30409895 
38. Kim JS, Kaufman J, Patel SS, Manco-Johnson M, Di Paola J, da Cruz EM. Antiplatelet Effect of Ketorolac in Children After Congenital Cardiac Surgery. World J Pediatr Congenit Heart Surg. 2018;9(6):651-658. PMID:30322371 
39. Fisher MH, Di Paola J. Genomics and transcriptomics of megakaryocytes and platelets: Implications for health and disease. Res Pract Thromb Haemost. 2018;2(4):630-639. PMCID:PMC6178711  PMID:30349880 
40. Smith N, Bornikova L, Noetzli L, Guglielmone H, Minoldo S, Backos DS, Jacobson L, Thornburg CD, Escobar M, White-Adams TC, Wolberg AS, Manco-Johnson M, Di Paola J. Identification and characterization of novel mutations implicated in congenital fibrinogen disorders. Res Pract Thromb Haemost. 2018;2(4):800-811. PMCID:PMC6178649  PMID:30349899 
41. Pluthero FG, Di Paola J, Carcao MD, Kahr WHA. NBEAL2 mutations and bleeding in patients with gray platelet syndrome. Platelets. 2018;29(6):632-635. PMID:29869935 
42. Link KG, Stobb MT, Di Paola J, Neeves KB, Fogelson AL, Sindi SS, Leiderman K. A local and global sensitivity analysis of a mathematical model of coagulation and platelet deposition under flow. PLoS One. 2018;13(7):e0200917. PMCID:PMC6062055  PMID:30048479 
43. Lavin M, Aguila S, Dalton N, Nolan M, Byrne M, Ryan K, White B, O'Connell NM, O'Sullivan JM, Di Paola J, James PD, O'Donnell JS. Significant gynecological bleeding in women with low von Willebrand factor levels. Blood Adv. 2018;2(14):1784-1791. PMCID:PMC6058240  PMID:30042144 
44. Shan Z, Liu X, Chen Y, Wang M, Gao YR, Xu L, Dar WA, Lee CG, Elias JA, Castillo PD, Di Paola J, Ju C. Chitinase 3-like-1 promotes intrahepatic activation of coagulation through induction of tissue factor in mice. Hepatology. 2018;67(6):2384-2396. PMCID:PMC5992002  PMID:29251791 
45. Ng CJ, Di Paola J. von Willebrand Disease: Diagnostic Strategies and Treatment Options. Pediatr Clin North Am. 2018;65(3):527-541. PMID:29803281 
46. Law LA, Graham DK, Di Paola J, Branchford BR. GAS6/TAM Pathway Signaling in Hemostasis and Thrombosis. Front Med (Lausanne). 2018;5:137. PMCID:PMC5954114  PMID:29868590 
47. Ng CJ, McCrae KR, Ashworth K, Sosa LJ, Betapudi V, Manco-Johnson MJ, Liu A, Dong JF, Chung D, White-Adams TC, López JA, Di Paola J. Effects of anti-β2GPI antibodies on VWF release from human umbilical vein endothelial cells and ADAMTS13 activity. Res Pract Thromb Haemost. 2018;2(2):380-389. PMCID:PMC5974922  PMID:30046742 
48. Branchford BR, Stalker TJ, Law L, Acevedo G, Sather S, Brzezinski C, Wilson KM, Minson K, Lee-Sherick AB, Davizon-Castillo P, Ng C, Zhang W, Neeves KB, Lentz SR, Wang X, Frye SV, Shelton Earp H 3rd, DeRyckere D, Brass LF, Graham DK, Di Paola JA. The small-molecule MERTK inhibitor UNC2025 decreases platelet activation and prevents thrombosis. J Thromb Haemost. 2018;16(2):352-363. PMCID:PMC5858881  PMID:29045015 
49. Lehmann M, Ashworth K, Manco-Johnson M, Di Paola J, Neeves KB, Ng CJ. Evaluation of a microfluidic flow assay to screen for von Willebrand disease and low von Willebrand factor levels. J Thromb Haemost. 2018;16(1):104-115. PMCID:PMC5794217  PMID:29064615 
50. Lavin M, Aguila S, Schneppenheim S, Dalton N, Jones KL, O'Sullivan JM, O'Connell NM, Ryan K, White B, Byrne M, Rafferty M, Doyle MM, Nolan M, Preston RJS, Budde U, James P, Di Paola J, O'Donnell JS. Novel insights into the clinical phenotype and pathophysiology underlying low VWF levels. Blood. 2017;130(21):2344-2353. PMCID:PMC5881608  PMID:28916584 
51. McGraw MD, Osborne CM, Mastej EJ, Di Paola JA, Anderson DR, Holmes WW, Paradiso DC, Garlick RB, Hendry-Hofer TB, Rancourt RC, Smith RW, Burns C, Roe GB, Rioux JS, White CW, Veress LA. Editor's Highlight: Pulmonary Vascular Thrombosis in Rats Exposed to Inhaled Sulfur Mustard. Toxicol Sci. 2017;159(2):461-469. PMCID:PMC5837673  PMID:28962529 
52. Miller CH, Boylan B, Shapiro AD, Lentz SR, Wicklund BM, Hemophilia Inhibitor Research Study Investigators.. Limit of detection and threshold for positivity of the Centers for Disease Control and Prevention assay for factor VIII inhibitors. J Thromb Haemost. 2017;15(10):1971-1976. PMCID:PMC5716470  PMID:28795528 
53. Maloney JP, Branchford BR, Brodsky GL, Cosmic MS, Calabrese DW, Aquilante CL, Maloney KW, Gonzalez JR, Zhang W, Moreau KL, Wiggins KL, Smith NL, Broeckel U, Di Paola J. The ENTPD1 promoter polymorphism -860 A > G (rs3814159) is associated with increased gene transcription, protein expression, CD39/NTPDase1 enzymatic activity, and thromboembolism risk. FASEB J. 2017;31(7):2771-2784. PMCID:PMC6137499  PMID:28302652 
54. Kastelowitz N, Tamura R, Onasoga A, Stalker TJ, White OR, Brown PN, Brodsky GL, Brass LF, Branchford BR, Di Paola J, Yin H. Peptides derived from MARCKS block coagulation complex assembly on phosphatidylserine. Sci Rep. 2017;7(1):4275. PMCID:PMC5487340  PMID:28655899 
55. Davizon-Castillo P, Di Paola J. Tamoxifen Suppresses Platelet Activation-Supported Angiogenesis and Metastasis. Arterioscler Thromb Vasc Biol. 2017;37(4):611-612. PMCID:PMC5873979  PMID:28330945 
56. Moreira DC, Di Paola J. Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome: reply. J Thromb Haemost. 2017;15(4):827-828. PMID:28182327 
57. Schoeman RM, Rana K, Danes N, Lehmann M, Di Paola JA, Fogelson AL, Leiderman K, Neeves KB. A microfluidic model of hemostasis sensitive to platelet function and coagulation. Cell Mol Bioeng. 2017;10(1):3-15. PMCID:PMC5435378  PMID:28529666 
58. Moreira DC, Ng CJ, Quinones R, Liang X, Chung DW, Di Paola J. Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome. J Thromb Haemost. 2016;14(12):2353-2355. PMCID:PMC6553946  PMID:27622772 
59. Gupta S, Heiman M, Duncan N, Hinckley J, Di Paola J, Shapiro AD. Variable bleeding phenotype in an Amish pedigree with von Willebrand disease. Am J Hematol. 2016;91(10):E431-5. PMCID:PMC5031525  PMID:27414491 
60. Boylan B, Rice AS, Neff AT, Manco-Johnson MJ, Kempton CL, Miller CH, Hemophilia Inhibitor Research Study Investigators.. Survey of the anti-factor IX immunoglobulin profiles in patients with hemophilia B using a fluorescence-based immunoassay. J Thromb Haemost. 2016;14(10):1931-1940. PMCID:PMC5083216  PMID:27501440 
61. Deng W, Xu Y, Chen W, Paul DS, Syed AK, Dragovich MA, Liang X, Zakas P, Berndt MC, Di Paola J, Ware J, Lanza F, Doering CB, Bergmeier W, Zhang XF, Li R. Platelet clearance via shear-induced unfolding of a membrane mechanoreceptor. Nat Commun. 2016;7:12863. PMCID:PMC5052631  PMID:27670775 
62. Guglielmone HA, Smith N, Minoldo S, Jarchum GD, White-Adams T, Di Paola J. Platelet abnormalities in a family with von Willebrand disease type 2B (V1316M) and association with bleeding score. Haemophilia. 2016;22(5):e455-8. PMID:27385556 
63. White-Adams TC, Ng CJ, Jacobi PM, Haberichter SL, Di Paola JA. Mutations in the D'D3 region of VWF traditionally associated with type 1 VWD lead to quantitative and qualitative deficiencies of VWF. Thromb Res. 2016;145:112-8. PMCID:PMC5951169  PMID:27533707 
64. Tomberg K, Khoriaty R, Westrick RJ, Fairfield HE, Reinholdt LG, Brodsky GL, Davizon-Castillo P, Ginsburg D, Di Paola J. Spontaneous 8bp Deletion in Nbeal2 Recapitulates the Gray Platelet Syndrome in Mice. PLoS One. 2016;11(3):e0150852. PMCID:PMC4780761  PMID:26950939 
65. Othman M, Kaur H, Favaloro EJ, Lillicrap D, Di Paola J, Harrison P, Gresele P, Subcommittees on von Willebrand Disease and Platelet Physiology.. Platelet type von Willebrand disease and registry report: communication from the SSC of the ISTH. J Thromb Haemost. 2016;14(2):411-4. PMID:26882161 
66. Di Paola J. Paris-Trousseau: evidence keeps pointing to FLI1. Blood. 2015;126(17):1973-4. PMID:26494917 
67. Kirkpatrick G, Noetzli L, Di Paola J, Porter CC. ETV6 mutations define a new cancer predisposition syndrome. Oncotarget. 2015;6(19):16830-1. PMCID:PMC4627276  PMID:26219557 
68. Chen J, Hinckley JD, Haberichter S, Jacobi P, Montgomery R, Flood VH, Wong R, Interlandi G, Chung DW, López JA, Di Paola J. Variable content of von Willebrand factor mutant monomer drives the phenotypic variability in a family with von Willebrand disease. Blood. 2015;126(2):262-9. PMCID:PMC4497966  PMID:26019279 
69. Branchford BR, Ng CJ, Neeves KB, Di Paola J. Microfluidic technology as an emerging clinical tool to evaluate thrombosis and hemostasis. Thromb Res. 2015;136(1):13-9. PMCID:PMC4910695  PMID:26014643 
70. Bodó I, Eikenboom J, Montgomery R, Patzke J, Schneppenheim R, Di Paola J, von Willebrand factor Subcommittee of the Standardization and Scientific Committee of the International Society for Thrombosis and Haemostasis.. Platelet-dependent von Willebrand factor activity. Nomenclature and methodology: communication from the SSC of the ISTH. J Thromb Haemost. 2015;13(7):1345-50. PMCID:PMC5576173  PMID:25858564 
71. Boylan B, Rice AS, De Staercke C, Eyster ME, Yaish HM, Knoll CM, Bean CJ, Miller CH, Hemophilia Inhibitor Research Study Investigators.. Evaluation of von Willebrand factor phenotypes and genotypes in Hemophilia A patients with and without identified F8 mutations. J Thromb Haemost. 2015;13(6):1036-42. PMCID:PMC4512234  PMID:25780857 
72. Noetzli L, Lo RW, Lee-Sherick AB, Callaghan M, Noris P, Savoia A, Rajpurkar M, Jones K, Gowan K, Balduini C, Pecci A, Gnan C, De Rocco D, Doubek M, Li L, Lu L, Leung R, Landolt-Marticorena C, Hunger S, Heller P, Gutierrez-Hartmann A, Xiayuan L, Pluthero FG, Rowley JW, Weyrich AS, Kahr WHA, Porter CC, Di Paola J. Germline mutations in ETV6 are associated with thrombocytopenia, red cell macrocytosis and predisposition to lymphoblastic leukemia. Nat Genet. 2015;47(5):535-538. PMCID:PMC4631613  PMID:25807284 
73. Ng C, Motto DG, Di Paola J. Diagnostic approach to von Willebrand disease. Blood. 2015;125(13):2029-37. PMCID:PMC4375103  PMID:25712990 
74. Boylan B, Rice AS, Dunn AL, Tarantino MD, Brettler DB, Barrett JC, Miller CH, Hemophilia Inhibitor Research Study Investigators., Hemophilia Inhibitor Research Study Investigators.. Characterization of the anti-factor VIII immunoglobulin profile in patients with hemophilia A by use of a fluorescence-based immunoassay. J Thromb Haemost. 2015;13(1):47-53. PMCID:PMC4383171  PMID:25354263 
75. Hinckley J, Di Paola J. Genetic basis of congenital platelet disorders. Hematology Am Soc Hematol Educ Program. 2014;2014(1):337-42. PMID:25696876 
76. Gonzalez-Alegre P, Di Paola J, Wang K, Fabbro S, Yu HC, Shaikh TH, Darbro BW, Bassuk AG. Evaluating Familial Essential Tremor with Novel Genetic Approaches: Is it a Genotyping or Phenotyping Issue? Tremor Other Hyperkinet Mov (N Y). 2014;4:258. PMCID:PMC4219111  PMID:25374765 
77. Aleman MM, Byrnes JR, Wang JG, Tran R, Lam WA, Di Paola J, Mackman N, Degen JL, Flick MJ, Wolberg AS. Factor XIII activity mediates red blood cell retention in venous thrombi. J Clin Invest. 2014;124(8):3590-600. PMCID:PMC4109540  PMID:24983320 
78. Di Paola J. Understanding congenital platelet disorders. Clin Adv Hematol Oncol. 2014;12(7):461-3. PMID:25322327 
79. Branchford BR, Ruegg K, Villalobos-Menuey E, Jacobson LJ, Di Paola JA, Manco-Johnson M. FVIII/VWF ratio is not a reliable predictor of VWD in children. Pediatr Blood Cancer. 2014;61(5):936-9. PMID:24281894 
80. Boulden Warren B, Di Paola JA. Platelets join the world of "Omics". Blood. 2014;123(16):2446-7. PMID:24744250 
81. Soucie JM, Miller CH, Kelly FM, Payne AB, Creary M, Bockenstedt PL, Kempton CL, Manco-Johnson MJ, Neff AT, Haemophilia Inhibitor Research Study Investigators.. A study of prospective surveillance for inhibitors among persons with haemophilia in the United States. Haemophilia. 2014;20(2):230-7. PMCID:PMC4519821  PMID:24261612 
82. Noetzli L, Sanz PG, Brodsky GL, Hinckley JD, Giugni JC, Giannaula RJ, Gonzalez-Alegre P, Di Paola J. A novel mutation in PLP1 causes severe hereditary spastic paraplegia type 2. Gene. 2014;533(1):447-50. PMID:24103481 
83. Zhang W, McIver AL, Stashko MA, DeRyckere D, Branchford BR, Hunter D, Kireev D, Miley MJ, Norris-Drouin J, Stewart WM, Lee M, Sather S, Zhou Y, Di Paola JA, Machius M, Janzen WP, Earp HS, Graham DK, Frye SV, Wang X. Discovery of Mer specific tyrosine kinase inhibitors for the treatment and prevention of thrombosis. J Med Chem. 2013;56(23):9693-700. PMCID:PMC3962266  PMID:24219778 
84. Onasoga-Jarvis AA, Leiderman K, Fogelson AL, Wang M, Manco-Johnson MJ, Di Paola JA, Neeves KB. The effect of factor VIII deficiencies and replacement and bypass therapies on thrombus formation under venous flow conditions in microfluidic and computational models. PLoS One. 2013;8(11):e78732. PMCID:PMC3827262  PMID:24236042 
85. Kahr WH, Lo RW, Li L, Pluthero FG, Christensen H, Ni R, Vaezzadeh N, Hawkins CE, Weyrich AS, Di Paola J, Landolt-Marticorena C, Gross PL. Abnormal megakaryocyte development and platelet function in Nbeal2(-/-) mice. Blood. 2013;122(19):3349-58. PMCID:PMC3953091  PMID:23861251 
86. Mannuccio Mannucci P, Kyrle PA, Schulman S, Di Paola J, Schneppenheim R, Cox Gill J. Prophylactic efficacy and pharmacokinetically guided dosing of a von Willebrand factor/factor VIII concentrate in adults and children with von Willebrand's disease undergoing elective surgery: a pooled and comparative analysis of data from USA and European Union clinical trials. Blood Transfus. 2013;11(4):533-40. PMCID:PMC3827397  PMID:23736911 
87. Hinckley JD, Abbott D, Burns TL, Heiman M, Shapiro AD, Wang K, Di Paola J. Quantitative trait locus linkage analysis in a large Amish pedigree identifies novel candidate loci for erythrocyte traits. Mol Genet Genomic Med. 2013;1(3):131-141. PMCID:PMC3775389  PMID:24058921 
88. Miller CH, Rice AS, Boylan B, Shapiro AD, Lentz SR, Wicklund BM, Kelly FM, Soucie JM, Hemophilia Inhibitor Research Study Investigators.. Comparison of clot-based, chromogenic and fluorescence assays for measurement of factor VIII inhibitors in the US Hemophilia Inhibitor Research Study. J Thromb Haemost. 2013;11(7):1300-9. PMCID:PMC4477744  PMID:23601690 
89. Di Paola J. SHPing in different directions in platelet production. Blood. 2013;121(20):4018-9. PMID:23682031 
90. Flood VH, Friedman KD, Gill JC, Haberichter SL, Christopherson PA, Branchford BR, Hoffmann RG, Abshire TC, Dunn AL, Di Paola JA, Hoots WK, Brown DL, Leissinger C, Lusher JM, Ragni MV, Shapiro AD, Montgomery RR. No increase in bleeding identified in type 1 VWD subjects with D1472H sequence variation. Blood. 2013;121(18):3742-4. PMCID:PMC3643771  PMID:23520336 
91. Di Paola J. Limited physical therapy utilization protocol does not affect impairment and disability in Workers' Compensation patients after rotator cuff repair: a short-term follow-up study. J Shoulder Elbow Surg. 2013;22(3):409-17. PMID:22939405 
92. Branchford BR, Monahan PE, Di Paola J. New developments in the treatment of pediatric hemophilia and bleeding disorders. Curr Opin Pediatr. 2013;25(1):23-30. PMID:23274430 
93. Ahmad S, Ahmad A, Rancourt RC, Neeves KB, Loader JE, Hendry-Hofer T, Di Paola J, Reynolds SD, White CW. Tissue factor signals airway epithelial basal cell survival via coagulation and protease-activated receptor isoforms 1 and 2. Am J Respir Cell Mol Biol. 2013;48(1):94-104. PMCID:PMC3547080  PMID:23065128 
94. Neeves KB, Onasoga AA, Hansen RR, Lilly JJ, Venckunaite D, Sumner MB, Irish AT, Brodsky G, Manco-Johnson MJ, Di Paola JA. Sources of variability in platelet accumulation on type 1 fibrillar collagen in microfluidic flow assays. PLoS One. 2013;8(1):e54680. PMCID:PMC3552855  PMID:23355889 
95. Marchi R, Walton BL, McGary CS, Lin FC, Ma AD, Pawlinski R, Mackman N, Campbell RA, Di Paola J, Wolberg AS. Dysregulated coagulation associated with hypofibrinogenaemia and plasma hypercoagulability: implications for identifying coagulopathic mechanisms in humans. Thromb Haemost. 2012;108(3):516-26. PMCID:PMC3627352  PMID:22836883 
96. Miller CH, Platt SJ, Rice AS, Kelly F, Soucie JM, Hemophilia Inhibitor Research Study Investigators.. Validation of Nijmegen-Bethesda assay modifications to allow inhibitor measurement during replacement therapy and facilitate inhibitor surveillance. J Thromb Haemost. 2012;10(6):1055-61. PMCID:PMC4477703  PMID:22435927 
97. Miller CH, Benson J, Ellingsen D, Driggers J, Payne A, Kelly FM, Soucie JM, Craig Hooper W, Hemophilia Inhibitor Research Study Investigators.. F8 and F9 mutations in US haemophilia patients: correlation with history of inhibitor and race/ethnicity. Haemophilia. 2012;18(3):375-82. PMID:22103590 
98. Di Paola J. Disability, impairment, and physical therapy utilization after arthroscopic partial meniscectomy in patients receiving workers' compensation. J Bone Joint Surg Am. 2012;94(6):523-30. PMID:22438001 
99. Branchford BR, Di Paola J. Making a diagnosis of VWD. Hematology Am Soc Hematol Educ Program. 2012;2012:161-7. PMCID:PMC5873588  PMID:23233576 
100. Hansen RR, Tipnis AA, White-Adams TC, Di Paola JA, Neeves KB. Characterization of collagen thin films for von Willebrand factor binding and platelet adhesion. Langmuir. 2011;27(22):13648-58. PMID:21967679 
101. Di Paola J, Johnson J. Thrombocytopenias due to gray platelet syndrome or THC2 mutations. Semin Thromb Hemost. 2011;37(6):690-7. PMID:22102272 
102. Di Paola J, Lethagen S, Gill J, Mannucci P, Manco-Johnson M, Bernstein J, Nichols WL, Bergman GE. Presurgical pharmacokinetic analysis of a von Willebrand factor/factor VIII (VWF/FVIII) concentrate in patients with von Willebrand's disease (VWD) has limited value in dosing for surgery. Haemophilia. 2011;17(5):752-8. PMID:21689209 
103. Kahr WH, Hinckley J, Li L, Schwertz H, Christensen H, Rowley JW, Pluthero FG, Urban D, Fabbro S, Nixon B, Gadzinski R, Storck M, Wang K, Ryu GY, Jobe SM, Schutte BC, Moseley J, Loughran NB, Parkinson J, Weyrich AS, Di Paola J. Mutations in NBEAL2, encoding a BEACH protein, cause gray platelet syndrome. Nat Genet. 2011;43(8):738-40. PMCID:PMC6050511  PMID:21765413 
104. Fabbro S, Kahr WH, Hinckley J, Wang K, Moseley J, Ryu GY, Nixon B, White JG, Bair T, Schutte B, Di Paola J. Homozygosity mapping with SNP arrays confirms 3p21 as a recessive locus for gray platelet syndrome and narrows the interval significantly. Blood. 2011;117(12):3430-4. PMCID:PMC3069679  PMID:21263149 
105. Flood VH, Gill JC, Morateck PA, Christopherson PA, Friedman KD, Haberichter SL, Branchford BR, Hoffmann RG, Abshire TC, Di Paola JA, Hoots WK, Leissinger C, Lusher JM, Ragni MV, Shapiro AD, Montgomery RR. Common VWF exon 28 polymorphisms in African Americans affecting the VWF activity assay by ristocetin cofactor. Blood. 2010;116(2):280-6. PMCID:PMC2910611  PMID:20231421 
106. Monahan PE, Di Paola J. Recombinant factor IX for clinical and research use. Semin Thromb Hemost. 2010;36(5):498-509. PMID:20632248 
107. James P, Di Paola J. The application of genetics to inherited bleeding disorders. Haemophilia. 2010;16 Suppl 5:35-9. PMID:20590854 
108. Björkman S, Blanchette VS, Fischer K, Oh M, Spotts G, Schroth P, Fritsch S, Patrone L, Ewenstein BM, Advate Clinical Program Group., Collins PW. Comparative pharmacokinetics of plasma- and albumin-free recombinant factor VIII in children and adults: the influence of blood sampling schedule on observed age-related differences and implications for dose tailoring. J Thromb Haemost. 2010;8(4):730-6. PMCID:PMC2855866  PMID:20398185 
109. Collins PW, Blanchette VS, Fischer K, Björkman S, Oh M, Fritsch S, Schroth P, Spotts G, Astermark J, Ewenstein B, rAHF-PFM Study Group.. Break-through bleeding in relation to predicted factor VIII levels in patients receiving prophylactic treatment for severe hemophilia A. J Thromb Haemost. 2009;7(3):413-20. PMID:19143924 
110. Young G, Ebbesen LS, Viuff D, Di Paola J, Konkle BA, Negrier C, Pasi J, Ingerslev J. Evaluation of thromboelastography for monitoring recombinant activated factor VII ex vivo in haemophilia A and B patients with inhibitors: a multicentre trial. Blood Coagul Fibrinolysis. 2008;19(4):276-82. PMID:18469548 
111. Hoots WK, Ebbesen LS, Konkle BA, Auerswald GK, Roberts HR, Weatherall J, Ferran JM, Ljung RC, Novoseven (F7HAEM-1505) Investigators.. Secondary prophylaxis with recombinant activated factor VII improves health-related quality of life of haemophilia patients with inhibitors. Haemophilia. 2008;14(3):466-75. PMID:18282155 
112. Sharthkumar A, Greist A, Di Paola J, Winay J, Roberson C, Heiman M, Herbert S, Parameswaran R, Shapiro A. Biologic response to subcutaneous and intranasal therapy with desmopressin in a large Amish kindred with Type 2M von Willebrand disease. Haemophilia. 2008;14(3):539-48. PMID:18312368 
113. Abbott D, Di Paola J. VWD type 1: a calculated diagnosis. Blood. 2008;111(8):3919-20. PMID:18434968 
114. Jobe SM, Wilson KM, Leo L, Raimondi A, Molkentin JD, Lentz SR, Di Paola J. Critical role for the mitochondrial permeability transition pore and cyclophilin D in platelet activation and thrombosis. Blood. 2008;111(3):1257-65. PMCID:PMC2214770  PMID:17989312 
115. Di Paola J, Smith MP, Klamroth R, Mannucci PM, Kollmer C, Feingold J, Kessler C, Pollmann H, Morfini M, Udata C, Rothschild C, Hermans C, Janco R. ReFacto and Advate: a single-dose, randomized, two-period crossover pharmacokinetics study in subjects with haemophilia A. Haemophilia. 2007;13(2):124-30. PMID:17286764 
116. Di Paola J, Aledort L, Britton H, Carcao M, Grabowski E, Hutter J, Journeycake J, Kempton C, Leissinger C. Application of current knowledge to the management of bleeding events during immune tolerance induction. Haemophilia. 2006;12(6):591-7. PMID:17083508 
117. Jobe SM, Leo L, Eastvold JS, Dickneite G, Ratliff TL, Lentz SR, Di Paola J. Role of FcRgamma and factor XIIIA in coated platelet formation. Blood. 2005;106(13):4146-51. PMCID:PMC1895237  PMID:16105983 
118. Shafagh H, Soltani MA, Douvoyiannis M, Widness J, Di Paola J, Yadav N, Jatzlau A, Pohl J, Weir M. Index of suspicion. Pediatr Rev. 2005;26(12):461-6. PMID:16327027 
119. Di Paola J. Determinants of bleeding severity in von Willebrand disease. Curr Hematol Rep. 2005;4(5):345-9. PMID:16131434 
120. Di Paola J, Jugessur A, Goldman T, Reiland J, Tallman D, Sayago C, Murray JC. Platelet glycoprotein I(b)alpha and integrin alpha2 beta1 polymorphisms: gene frequencies and linkage disequilibrium in a population diversity panel. J Thromb Haemost. 2005;3(7):1511-21. PMID:15978109 
121. Shapiro AD, Di Paola J, Cohen A, Pasi KJ, Heisel MA, Blanchette VS, Abshire TC, Hoots WK, Lusher JM, Negrier C, Rothschild C, Roth DA. The safety and efficacy of recombinant human blood coagulation factor IX in previously untreated patients with severe or moderately severe hemophilia B. Blood. 2005;105(2):518-25. PMID:15383463 
122. Di Paola J. Product selection issues in the management of hemophilia B. Blood Coagul Fibrinolysis. 2004;15 Suppl 2:S17-8. PMID:15322454 
123. Jobe S, Donohoue P, Di Paola J. Deep venous thrombosis and Turner syndrome. J Pediatr Hematol Oncol. 2004;26(4):272. PMID:15087960 
124. Di Paola J, Goldman T, Qian Q, Patil SR, Schutte BC. Breakpoint of a balanced translocation (X:14) (q27.1;q32.3) in a girl with severe hemophilia B maps proximal to the factor IX gene. J Thromb Haemost. 2004;2(3):437-40. PMID:15009460 
125. Leo L, Di Paola J, Judd BA, Koretzky GA, Lentz SR. Role of the adapter protein SLP-76 in GPVI-dependent platelet procoagulant responses to collagen. Blood. 2002;100(8):2839-44. PMID:12351393 
126. Di Paola JA, Buchanan GR. Immune thrombocytopenic purpura. Pediatr Clin North Am. 2002;49(5):911-28. PMID:12430619 
127. Di Paola J, Nugent D, Young G. Current therapy for rare factor deficiencies. Haemophilia. 2001;7 Suppl 1:16-22. PMID:11240614 
128. Di Paola J, Federici AB, Mannucci PM, Canciani MT, Kritzik M, Kunicki TJ, Nugent D. Low platelet alpha2beta1 levels in type I von Willebrand disease correlate with impaired platelet function in a high shear stress system. Blood. 1999;93(11):3578-82. PMID:10339461 
129. Doncel GF, Di Paola JA, Lustig L. Sequential study of the histopathology and cellular and humoral immune response during the development of an autoimmune orchitis in Wistar rats. Am J Reprod Immunol. 1989;20(2):44-51. PMID:2803528